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Frataxin and mitochondrial FeS cluster biogenesis.

ABSTRACT: Friedreich ataxia is an inherited neurodegenerative disease caused by frataxin deficiency. Frataxin is a conserved mitochondrial protein that plays a role in FeS cluster assembly in mitochondria. FeS clusters are modular cofactors that perform essential functions throughout the cell. They are synthesized by a multistep and multisubunit mitochondrial machinery that includes the scaffold protein Isu for assembling a protein-bound FeS cluster intermediate. Frataxin interacts with Isu, iron, and the cysteine desulfurase Nfs1, which supplies sulfide, thus placing it at the center of mitochondrial FeS cluster biosynthesis.

SUBMITTER: Stemmler TL

PROVIDER: S-EPMC2930671 | biostudies-literature | 2010 Aug

REPOSITORIES: biostudies-literature

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Frataxin and mitochondrial FeS cluster biogenesis.

Stemmler Timothy L TL Lesuisse Emmanuel E Pain Debkumar D Dancis Andrew A

The Journal of biological chemistry 20100603 35

Friedreich ataxia is an inherited neurodegenerative disease caused by frataxin deficiency. Frataxin is a conserved mitochondrial protein that plays a role in FeS cluster assembly in mitochondria. FeS clusters are modular cofactors that perform essential functions throughout the cell. They are synthesized by a multistep and multisubunit mitochondrial machinery that includes the scaffold protein Isu for assembling a protein-bound FeS cluster intermediate. Frataxin interacts with Isu, iron, and the ...[more]

PMID: 20522547

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